[ myoclonic epilepsy in infancy: about 37 cases]

ABSTRACT

The benign myoclonic epilepsy in infancy [BMEN] is described by Dravet in 1981. It is rare.Defined by the supervene of myoclonic attacks, without other types of attacks in normal infancy aged from 4 months to 3 years old. It is classified among the genaralized idiopathic epilepsies. The purpose of our study is to rate epidemiologic, clinic, electric, therapeutic and evolutive data about BMEN. Our study was retrospective, between 1997 and 2002, based on a card of exploitation including the criteria of inclusion according the definition of this type of epilepsy in the international classification of the epilepsy 1989. We collected 37 cases of BMEN let 1.2% of all the epilepsies. The female sex predominates slightly [55%], the mean age at the begining has been 17 months,the mean age at the consultation has been more than 24 months . About the past, all the children were born at full term, the medicalized delivery noticed in 98% of the cases, normal psychomotor growth in all the cases, the consanguinity represents 11 % of the cases and 19% of similar cases in the family.Clinically, the attacks were myoclonic and recurrent in 30 cases [81%], generalized in 30 cases [81%] and partial in 7 cases [5cases of bending of the head on the trunk, 2 cases at the level of the upper limb] and fall in 6 cases [16%]. The neurologic examination was normal in all the children. The electric signs consist in peaks-waves, and electroencephalographic peaks waves generalized in 43%, with photosensitiveness. The cerebral computerized tomography realized in 7 nursling was normal. The treatement has been based on the valprorate of sodium as monotherapy in the majority of the cases with good evolution in 90% of the cases.the prognosis is usually good. If the myoclonies resist to the valporate of sodium, the adjunction of a Benzodiazepine or ethoxumide may be suggested.The genetic data are unknown.Hence, the signifiant role of the molecular biology